GUIDANCE STATEMENTS PBC AASLD 2018:

1. Diagnosis :- The diagnosis of PBC can be established when two of the following three criteria are met:
• Biochemical evidence of cholestasis based on ALP elevation.
• Presence of AMA, or other PBC-specific autoantibodies, including sp100 or gp210, if AMA is negative.
• Histologic evidence of non suppurative destructive cholangitis and destruction of interlobular bile ducts.

2. The diagnosis of AMA-negative PBC does not require a liver biopsy if other criteria are met, including
cholestatic liver tests and PBC-specific autoantibodies such as sp100 or gp210.

3. Liver biopsy to rule out concomitant AIH or other liver disease should be considered in PBC patients
when the alanine aminotransferase activity is more than 5 times the upper limit of normal.

4. In cases of suspected PBC/AIH overlap, treatment should be targeted at the predominant histological
pattern of injury.

5.UDCA in a dose of 13 to 15 mg/kg/day orally is recommended for patients with PBC who have abnormal
liver enzyme values regardless of histologic stage.

6. For patients requiring bile acid sequestrants ( cholestyramine, chleseveloam, cholestipol ), UDCA should
be given at least 1 hour before or 4 hours after the bile acid sequestrant.

7. Biochemical response to UDCA

should be evaluated at 12 months
after treatment initiation to
determine whether patients should
be considered for second-line
therapy.

8. Patients who are inadequate

responders to UDCA (Table 1) should
be considered for treatment with
OCA, starting at 5 mg/day.

9. Fibrates can be considered as off-label alternatives for patients with PBC and inadequate response to
UDCA.

10.Use of OCA and fibrates is discouraged in patients with decompensated liver disease (Child Pugh-
Turcotte B or C).

11. Anion-exchange resins should be used as initial therapy for patients with PBC who have pruritus.

12. The following agents can be used for pruritus refractory to anion-exchange resins: a. Rifampicin 150
to 300 mg twice daily. b. Oral opiate antagonists such as naltrexone titrated to a dose of 50 mg daily. c.
Sertraline 75 to 100 mg daily.

13.Management of dry eyes can include the following: a. Artificial tears should be used initially. b.
Pilocarpine or cevimeline can be used in patients for whom symptoms are refractory to artificial tears. c.
Cyclosporine or lifitegrast ophthalmic emulsion can be used in those whose disease is refractory to other
agents, preferably under the supervision of an ophthalmologist.
14.The following therapies should be used for xerostomia and dysphagia: a. Over-the-counter saliva
substitutes can be tried. b. Pilocarpine or cevimeline can be used if patients remain symptomatic despite
saliva substitutes.

15. Patients with suspected cirrhosis should undergo endoscopic screening for varices at the time of
diagnosis.

16. Regular screening for hepatocellular carcinoma with cross-sectional imaging at 6-month intervals is
currently advised for men and patients with cirrhosis.

17.Patients with PBC should be provided 1,000 to 1,500 mg of calcium and 1,000 International Units of
vitamin D daily in the diet and as supplements if needed.

18. Bone disease:- Oral alendronate (70 mg weekly) or other effective bisphosphonates should be
considered if patients are osteoporotic. Oral bisphosphonates should be avoided if patients have acid
reflux or known varices.

19. Patients with elevated lipid levels and at risk for cardiovascular disease can be considered for lipid
lowering therapy.

20. Fat-soluble vitamin deficiencies should be treated with parenteral or water-soluble supplements.

21. Indications for liver transplantation for patients with PBC are similar to those with other forms of
chronic liver disease. Patients should be referred for liver transplant evaluation in the setting of
decompensated cirrhosis,
a. Model for End-Stage Liver Disease score of at least 15, total bilirubin greater than 6 mg/dL, or a
b. Mayo risk score greater than 7.8.
c. Severe intractable pruritus is an exceptional indication for liver transplantation.
Chronic fatigue is not an indication for transplant because this symptom is not universally reversible after
liver transplantation.